Primary aldosteronism — a multidimensional syndrome

Turcu, Adina F.; Yang, Jun; Vaidya, Anand

doi:10.1038/s41574-022-00730-2

Cited by 56 publications

(49 citation statements)

References 241 publications

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“…Variable expression of melanocortin type 2 receptors (MC2R) in primary aldosteronism ACTH stimulatory effects on AVS results vary greatly in part due to the variable expression of ACTH receptors, or MC2R, in APAs and PA micronodules compared to normal zona glomerulosa [32]. It has been shown that while APAs with ATPase somatic mutations demonstrate a high MC2R expression, those with KCNJ5 mutations tend to be poor in MC2R [1]. In the former case, this results in an increased ipsilateral aldosterone secretion, and in the latter, a relatively higher aldosterone secretion from the surrounding (and contralateral) cortex following ACTH stimulation [1].…”

Section: Classical Dichotomy Between Uniform Phenotypes Of Aldosteron...mentioning

confidence: 99%

“…Early case detection and adequate therapeutic management of primary aldosteronism (PA) are crucial to reducing the associated increased risk of metabolic and cardiovascular complications of PA [1][2][3]. In a classic dichotomous manner, PA was believed to be mostly secondary, in almost equal proportions, to either unilateral single aldosterone-producing adenomas (or aldosteronomas) (APA) or bilateral idiopathic hyperaldosteronism (IHA) [1,4,5]. Adrenal vein sampling (AVS) has been performed to distinguis between both etiologies and recommending surgical therapy in unilateral APA or medical therapy in bilateral IHA [4].…”

Section: Introductionmentioning

confidence: 99%

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Role of Adrenal Vein Sampling in Guiding Surgical Decision in Primary Aldosteronism

Younes,

Larose,

Bourdeau

et al. 2023

Exp Clin Endocrinol Diabetes

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Adrenal vein sampling (AVS) is recommended for subtyping primary aldosteronism (PA) to identify lateralized or bilateral sources of aldosterone excess, allowing for better decision-making in regard to medical or surgical management on a case-by-case basis. To date, no consensus exists on protocols to be used during AVS, especially concerning sampling techniques, the timing of sampling, and whether or not to use adrenocorticotropic hormone (ACTH) stimulation. Interpretation criteria for selectivity, lateralization, and contralateral suppression vary from one expert center to another, with some favoring strict cut-offs to others being more permissive. Clinical and biochemical post-operative outcomes can also be influenced by AVS criteria utilized to indicate surgical therapy.In this review, we reanalyze studies on AVS highlighting the recent pathological findings of frequent micronodular hyperplasia adjacent to a dominant aldosteronoma (APA) overlapping with bilateral idiopathic hyperaldosteronism (IHA) etiologies, as opposed to the less frequent unilateral single aldosteronoma. The variable expression of melanocortin type 2 receptors in the nodules and hyperplasia may explain the frequent discordance in lateralization ratios between unstimulated and ACTH- stimulated samples. We conclude that aldosterone values collected during simultaneous bilateral sampling, both at baseline and post-ACTH stimulation, are required to adequately evaluate selectivity, lateralization, and contralateral suppression during AVS, to better identify all patients with PA that can benefit from a surgical indication. Recommended cut-offs for each ratio are also presented.

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Section: Classical Dichotomy Between Uniform Phenotypes Of Aldosteron...mentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Role of Adrenal Vein Sampling in Guiding Surgical Decision in Primary Aldosteronism

Younes,

Larose,

Bourdeau

et al. 2023

Exp Clin Endocrinol Diabetes

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“…Primary aldosteronism is increasingly recognized as the most frequent cause of secondary hypertension, responsible for up to 20% of patients with resistant hypertension (1). This still largely underdiagnosed disease is secondary to two main presentations, with aldosterone secreting lateralized adenoma or bilateral adrenal hyperplasia (in 50-70% of cases).…”

Section: Introductionmentioning

confidence: 99%

“…This still largely underdiagnosed disease is secondary to two main presentations, with aldosterone secreting lateralized adenoma or bilateral adrenal hyperplasia (in 50-70% of cases). The precise pathophysiological triggering mechanisms leading to increased aldosterone secretion remain incompletely understood, but mutations of several somatic ion-channel genes are found in aldosteronomas and micronodules (1). In physiology, but also in disease, ACTH can regulate aldosterone secretion depending on the levels of its Gprotein coupled receptor (GPCR) called MC2R (2).…”

Section: Introductionmentioning

confidence: 99%

“…Aberrant expression of other GPCR can also be involved in aldosterone excess: GPCR can be eutopic (5-HT4R for serotonin), or ectopic (such as the glucose-dependent insulinotropic peptide receptor, beta-adrenergic receptor, vasopressin receptor or glucagon receptor) (2). LH/HCG GPCR (LHCGR) and GnRHR can also be ectopically expressed in adrenal adenomas or adrenal hyperplasia leading to hyperaldosteronism (1). Roughly 15 years ago, Zwermann et al reported that the majority of both normal (though at low amounts) and tumoral adrenal tissues (14 aldosterone producing adenomas) expressed LHCGR mRNA; however, only 3 were responsive to GnRH stimulation (3).…”

Section: Introductionmentioning

confidence: 99%

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HCG-responsive aldosteronoma with transient secretion during pregnancy confirmed through HCG-stimulated adrenal venous sampling

et al. 2023

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Primary aldosteronism can be regulated by the ectopic expression of G-protein coupled receptors in aldosteronomas or bilateral hyperplasias. We report a rare case of a young woman in whom 2 pregnancies were complicated by pre-eclampsia and 1 miscarriage. The transient primary aldosteronism during pregnancies suggested the possibility of HCG stimulated aberrant adrenal expression of LHCG receptor in her adrenal tissues. This was supported by increased aldosterone and renin suppression during 5-day HCG stimulation test outside of pregnancy. Following a second 5-day HCG stimulation test, bilateral simultaneous adrenal vein sampling identified a lateralized source of aldosterone from an 8 mm right adrenal nodule. A right laparoscopic adrenalectomy resulted in clinical and biochemical cure and allowed a further uneventful pregnancy a few years later. This case illustrates the indication to investigate for potential primary aldosteronism in woman with transient hypertension during pregnancy.

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